Article ID Journal Published Year Pages File Type
5724611 Journal of Cystic Fibrosis 2017 8 Pages PDF
Abstract

BackgroundExtra-oesophageal reflux (EOR) may lead to microaspiration in patients with cystic fibrosis (CF), a probable cause of deteriorating lung function. Successful clinical trials of ivacaftor highlight opportunities to understand EOR in a real world study.MethodsData from 12 patients with CF and the G551D mutation prescribed ivacaftor (150 mg bd) was collected at baseline, 6, 26 and 52 weeks. The changes in symptoms of EOR were assessed by questionnaire (reflux symptom index (RSI) and Hull airway reflux questionnaire (HARQ)).ResultsSix patients presented EOR at baseline (RSI > 13; median 13; range 2-29) and 5 presented airway reflux (HARQ > 13; median 12; range 3 to 33). Treatment with ivacaftor was associated with a significant reduction of EOR symptoms (P < 0 ∙ 04 versus baseline) denoted by the reflux symptom index and Hull airway reflux questionnaire.ConclusionIvacaftor treatment was beneficial for patients with symptoms of EOR, thought to be a precursor to microaspiration.

Related Topics
Health Sciences Medicine and Dentistry Pulmonary and Respiratory Medicine
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